SELECTIVE IgM DEFICIENCY
 
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SELECTIVE IgM DEFICIENCY

 

DEFINITION:

A primary immunodeficiency disorder characterized by a reduction in the production of IgM antibodies resulting in recurrent bacterial infections.

EPIDEMIOLOGY:

  • incidence: 1/1000
  • age of onset:
    • ?
  • risk factors:
    • familial - autosomal recessive
      • chrom.#: ?
      • gene: ?
    • M = F

PATHOGENESIS:

1. Background

  • B cells differentiate into plasma cells which secrete IgM

2. Defect

  • etiology unknown:
    • hypothesized that either insufficient T helper cell or increased T suppressor cell functions interfere with IgM-committed B cell differentiation

CLINICAL FEATURES:

1. Immunodeficiency Manifestations

  • 1. Respiratory
    • recurrent respiratory tract infections (chronic cough)
  • 2. Others
    • recurrent bacterial infections
    • recurrent sepsis and/or urinary tract infections

    • 2. Complications

  • 1. Autoimmune Disorders
    • hemolytic anemia
  • 2. Gastrointestinal Associations
    • Crohn's Disease
    • Lymphoid Nodular Hyperplasia
    • Whipple Disease
    • malabsorption
    • splenomegaly
  • 3. Others
    • atopy (eczema)

    • INVESTIGATIONS:

    1. Serum

  • 1. Humoral-Mediated
    • absent or reduced IgM levels
    • normal IgG and IgA levels
  • 2. Cell-Mediated
    • may be an abnormal T helper or T suppressor cell function

    • MANAGEMENT:

    1. Supportive

    • aggressive treatment with antibiotics
    • antibiotics and fresh frozen plasma infusions for severe infections

    2. Prognosis

    • patients tend to succumb to rapid hematogenous spread of bacterial infections

     

     

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